Fibromuscular dysplasia is non-inflammatory diseases. Unlike in adults, there is no biasness in the incidence of this disease based on gender in children. The accurate pathogenesis of the disease remains unclear and this may be one of the reasons for its underdiagnosis.  Further, delayed diagnosis in children may result in progression of this disease and the patient may experience refractory hypertension. The chances of successful treatment in patients with delayed diagnosis decreases.

There are various imaging techniques that are used for diagnosing FMD in children. These are magnetic resonance angiography (MRA), Doppler ultrasound (US), catheter-based angiography (angiography), and computed tomography angiography (CTA).

The authors of the study, while explaining the need of this study, wrote, “The main aim of the study was to compare the efficacy of imaging modalities which can allow for earlier and improved detection. Furthermore, an anatomical mapping of the location of lesions can help determine the best treatment modalities.”

The study involves 25 patients, which had non-inflammatory non-syndromic renovascular hypertension. The researchers evaluated the clinical presentation and imaging observations of CT, US, and MRI of the children who were diagnosed with FMD. 48% of the patients experienced headaches. 80% of the patients had a history of hypertension while the remaining 20% of the children were diagnosed with hypertension on presentation. Bruit was heard in only 12% of the patients. Fibromuscular dysplasia was unifocal with a single site or multiple sites. In 68% of the patients, there was an involvement of the main or first-order renal branch. 25% of the children had isolated distal lesions beyond second-order branches.

The study found that Ultrasound imaging was less sensitive than angiography. Further, magnetic resonance angiography is better as compared to ultrasound. However, computed tomography angiography has the best sensitivity as compared to catheter-based angiography. The study reveals that the distal vessel disease was only shown through angiography.

The study concludes that regardless of the finding observed through MRA, CTA, or US, catheter-based angiography should be performed as a part of the initial work-up.

Source

Louis R, Levy-Erez D, Cahill AM, Meyers KE. Imaging studies in pediatric fibromuscular dysplasia (FMD): a single-center experience. Pediatr Nephrol. 2018 Sep;33(9):1593-1599. doi: 10.1007/s00467-018-3983-6. Epub 2018 Jun 4. PMID: 29869115; PMCID: PMC